Erythrokeratodermia variabilis: Successful palliative treatment with acitretin

Erythrokeratodermia variabilis: successful palliative treatment with acitretin.

Sir, Erythrokeratodermia variabilis (EKV) is a rare autosomal dominant genodermatosis which is characterized by the coexistence of hyperkeratotic plaques and transient erythematous patches. Irregular, variably shaped erythematous macular patches may enlarge or regress over time. Concurrently, there are persistent, well-demarcated, geographic, hyperkeratotic plaques which are distributed on the ...

Erythrokeratodermia variabilis - variant with circumscribed variable erythema and periorificial fixed Bazex Dupré erythema.

Erythrokeratodermia represents a group of rare genetic diseases characterized through disorders of keratinization. Clinically, they are presenting themselves with erythematous and hyperkeratosic lesions that can be persistent or variable as to their aspect and localization. They were classified in erythrokeratodermia variabilis (EKV) and erythrokeratodermia symmetric progressive (EKSP). We are ...

Novel mutation in GJB4 gene (connexin 30.3) in a family with erythrokeratodermia variabilis.

© 2012 The Authors. doi: 10.2340/00015555-1436 Journal Compilation © 2012 Acta Dermato-Venereologica. ISSN 0001-5555 Erythrokeratodermia variabilis (EKV, MIM: 133200) is a rare autosomal dominant disorder characterized by the association of: (i) localized or generalized hyperkeratosis, and (ii) transient erythematous areas (1). Hyperkeratotic areas show well-demarcated, geographically outlined,...

Treatment Of Congenital Ichthyosis With Acitretin

We assessed the clinical efficacy, tolerability and safety of acitretin in a patient with ichthyosis. A newborn infant with ichthyosis who presented at birth with collodion baby appearance, was treated with acitretin. A moderate response to acitretin therapy (1 mg/kg/day) administered for 6 months was observed, with improvement in cutaneous lesions. Clinical improvement was achieved shortly aft...

An unusual missense mutation in the GJB3 gene resulting in severe erythrokeratodermia variabilis.